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Initial clinical manifestations and mid- and long-term results after surgical repair of double-chambered right ventricle in children and adults

Published online by Cambridge University Press:  01 June 2008

Ragiab Telagh ,
Vladimir Alexi-Meskishvili ,
Ronald Hetzer ,
Peter E. Lange ,
Felix Berger  and
Hashim Abdul-Khaliq
Ragiab Telagh
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany
Vladimir Alexi-Meskishvili
Affiliation:
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
Ronald Hetzer
Affiliation:
Department of Cardiothoracic and Vascular Surgery, Deutsches Herzzentrum Berlin, Berlin, Germany
Peter E. Lange
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany
Felix Berger
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany
Hashim Abdul-Khaliq*
Affiliation:
Department of Congenital Heart Disease, Deutsches Herzzentrum Berlin, Berlin, Germany Department of Paediatric Cardiology, Saarland University Hospital, Homburg/Saar, Germany
*
Correspondence to: Prof. Hashim Abdul-Khaliq, MD, PhD, Department of Paediatric Cardiology, Saarland University Hospital, 66421 Homburg/Saar, Germany. Tel: +49 6841-1628306; Fax: +49 6841-1628330; E-mail: [email protected]
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Abstract

Objective

By means of retrospective analysis of our institutional experience, we reviewed the clinical manifestation and outcomes of patients subsequent to surgical repair of double-chambered right ventricle.

Methods

Between 1988 and 2005, we performed surgical repair in 35 of 37 patients diagnosed with double-chambered right ventricle. The patients ranged in age from 4 to 69 years, with a mean of 21.3 years. Most presented in infancy, with initial manifestation of a short systolic murmur in 34 (92%) of all cases. Pressure gradients were measured invasively across the right ventricular outflow tract of between 30 and 140 mmHg, with a median of 60 mmHg. An associated ventricular septal defect was present in 26 patients (70%). Of the group, 4 patients were aged over 40 years, and 2 had previously undergone operative closure of a ventricular septal defect.

Results

The operative interval ranged from 2 months to 41 years, with a median of 9 years. In all, we resected muscular bundles and enlarged the right ventricular outflow tract. There was no hospital or late death. Median follow-up subsequent to surgery was 7 years, with a range from 0.4 to 11 years. No patient required further surgery to relieve any obstruction of the right ventricular outflow tract, nor long term medical therapy or pacing because of cardiac arrhythmia.

Conclusions

Surgical repair of a double-chambered right ventricle yields excellent haemodynamic and functional results over the mid to long term.

Keywords

Congenital heart diseasesobstructionmuscle bandcorrection
Type
Original Article
Information
Cardiology in the Young , Volume 18 , Issue 3 , June 2008 , pp. 268 - 274
Copyright
Copyright © Cambridge University Press 2008

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